Systemic Plasmacytosis and the Crow-Fukase Syndrome

Publisher Website
Google Scholar
Abstract
To the Editor.— In the November 1986 issue of the Archives, Watanabe et al1 described two patients with multiple cutaneous plasma cell infiltrates, polyclonal hypergammaglobulinemia, and generalized lymphadenopathy that showed the histologic features of Castleman's giant lymph node hyperplasia. I should like to highlight some similarities between this interesting syndrome and the Crow-Fukase syndrome (also called Shimpo's syndrome, Tatsuki syndrome, PEP syndrome, and POEMS syndrome).2 In the latter syndrome, hyperglobulinemia, which may be polyclonal or monoclonal, is associated with polyneuropathy and a variety of cutaneous and endocrinologic manifestations including edema, pigmentation, scleroderma, gynecomastia, and impotence. Only approximately 50% of cases have evidence of multiple myelomatosis. Generalized lymphadenopathy occurs in the majority of cases, and the lymph nodes often show Castleman's angiofollicular hyperplasia with sheets of mature plasma cells as described by Watanabe and colleagues in their two cases of systemic plasmacytosis. In the Crow-Fukase syndrome, the changes are